Malignant "triton" tumors: a clinicopathologic and immunohistochemical study of nine cases

Nine cases of malignant "triton" tumors, based on the coexistence of rhabdomyoblasts and Schwann cell elements, were analyzed clinicopathologically and immunocytochemically. All tumors were stained for myoglobin and S-100 protein by the immunoperoxidase technique. Six of the nine patients were in the third or fourth decade of life. Six cases were associated with von Recklinghausen's disease, and the tumors in two cases grew along nerve trunks. The malignant tumors showed a predilection for the thigh and buttock. Six of the seven deaths occurred within two years of the initial treatments. Strongly positive staining for S-100 protein was observed in three tumors, with transitional zones between the sarcomas and peripheral neurofibroma-like areas, as well as in two tumors composed predominantly of rhabdomyoblastic elements. In four other cases the tumors were only weakly positive for protein S-100. Intracytoplasmic myoglobin was present in all cases. Tumors composed predominantly of rhabdomyosarcomatous elements occurred in four patients, including two children with von Recklinghausen's disease. These results, considered with other findings, suggest that malignant "triton" tumors may not be as rare as previously believed.